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Klippel-Trenaunay syndrome

Definition

Klippel-Trenaunay syndrome is a group of findings consisting of:

• Multiple port wine stains or other blood vessel malformations, including dark spots of skin.
• Excessive growth of bones and soft tissue. This occurs most commonly in the legs, but may affect the arms, the face, head, or internal organs.
• Varicose veins that may be noted in early infancy. However, they are more likely to be seen later in childhood or adolescence. Bleeding from the rectum and blood in the urine may also occur.

Alternative Names

Klippel-Trenaunay-Weber syndrome; KTS; Angio-osteohypertrophy; Nevus varicosus osteohypertrophicus syndrome; Hemangiectasia hypertrophicans; Nevus verucosus hypertrophicans

Causes, incidence, and risk factors

Most cases of Klippel-Trenaunay syndrome occur for no apparent reason, although a few cases are thought to be passed down through families (inherited), possibly as an autosomal dominant trait.

Expectations (prognosis)

Most individuals with Klippel-Trenaunay syndrome do well despite their cosmetic appearance, although there can be associated psychological problems.

References

Garzon M, Huang J, Enjolras O, Frieden I. Vascular malformations. Part II: associated syndromes. J Am Acad Derm. April 2007; 56(4): 541-64.

Review Date:10/30/2007
Reviewed By:David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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